Adenoid cystic carcinoma (ACC) of Bartholin's gland is a rare variant and around 90 cases have been reported. Herein, the authors re port a locally advanced ACC of Bartholin's gland that was treated with neoadjuvant chemotherapy followed by right radical hemi-vulvectomy and reconstruction with a gracilis musculocutanous flap. There was no evidence of recurrence after three years of follow up.
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